Top

Published in:

Open Access 01-12-2019 | Azathioprine | Original Research

Late Onset of Neuromyelitis Optica Spectrum Disorders

Authors: Yara Dadalti Fragoso, Heloisa Helena Ruocco, Ronaldo Maciel Dias, Hideraldo Cabeça, Ricardo Gonçalves, Nise A. de Carvalho Sousa, Caroline Vieira Spessotto, Carlos Bernardo Tauil, Soniza Vieira Alves-Leon, Sidney Gomes, Marcus Vinicius M. Gonçalves, Suzana C. Nunes Machado, Andrea Anacleto, Eber Castro Correa, Maria Lucia V. Pimentel, Gutemberg Augusto C. Santos

Published in: Neurology and Therapy | Issue 2/2019

Abstract

Introduction

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune demyelinating disease of the central nervous system. NMOSD starting after the age of 50 years is considered a “late onset” (LO-NMOSD) and seems to be particularly aggressive. The objective of this paper is to present a series of 37 Brazilian patients with LO-NMOSD.

Methods

Retrospective data collection from medical records of patients with LO-NMOSD seen at 14 Brazilian specialized units.

Results

The ratio of women to men in the sample was 4.3 to 1. The patients were followed up for a median period of 4 years. Sex, age at disease onset, and ethnic background were not associated with the number of relapses or disability outcomes. Extensive longitudinal myelitis affected 86% of patients, while optic neuritis affected 70%, and brainstem syndromes were present in only 16% of these patients. Six patients are currently using some type of support for walking or are wheelchair-bound. Three have died. Therapeutic options for NMOSD were particularly complicated for these elderly patients, since medications for controlling NMOSD are, in essence, immunosuppressive. Long-term use of corticosteroids can be an issue when the patients have high blood pressure, diabetes mellitus, or dyslipidemia (conditions often seen in elderly individuals).

Conclusion

This series of LO-NMOSD cases highlights the importance of prompt diagnosis and treatment for these patients.

Akaishi T, Nakashima I, Sato DK, Takahashi T, Fujihara K. Neuromyelitis optica spectrum disorders. Neuroimaging Clin N Am. 2017;27(2):251–65. https://doi.org/10.1016/j.nic.2016.12.010.CrossRefPubMed

Weinshenker BG, Wingerchuk DM. Neuromyelitis spectrum disorders. Mayo Clin Proc. 2017;92(4):663–79. https://doi.org/10.1016/j.mayocp.2016.12.014.CrossRefPubMed

Wu Y, Zhong L, Geng J. Neuromyelitis optica spectrum disorder: pathogenesis, treatment, and experimental models. Mult Scler Relat Disord. 2018;27:412–8. https://doi.org/10.1016/j.msard.2018.12.002.CrossRefPubMed

Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2):177. https://doi.org/10.1212/wnl.0000000000001729.CrossRefPubMedPubMedCentral

Jasiak-Zatonska M, Kalinowska-Lyszczarz A, Michalak S, Kozubski W. The immunology of neuromyelitis optica—current knowledge, clinical implications, controversies and future perspectives. Int J Mol Sci. 2016;17(3):273. https://doi.org/10.3390/ijms17030273.CrossRefPubMedPubMedCentral

Han J, Yang MG, Zhu J, Jin T. Complexity and wide range of neuromyelitis optica spectrum disorders: more than typical manifestations. Neuropsychiatr Dis Treat. 2017;13:2653–60. https://doi.org/10.2147/NDT.S147360.CrossRefPubMedPubMedCentral

Wingerchuk DM. Neuromyelitis optica spectrum disorders. Continuum (Minneap Minn). 2010;16:105–21. https://doi.org/10.1212/01.CON.0000389937.69413.15.CrossRefPubMed

Mealy MA, Mossburg SE, Kim SH, et al. Long-term disability in neuromyelitis optica spectrum disorder with a history of myelitis is associated with age at onset, delay in diagnosis/preventive treatment, MRI lesion length and presence of symptomatic brain lesions. Mult Scler Relat Disord. 2018;28:64–8. https://doi.org/10.1016/j.msard.2018.12.011.CrossRefPubMedPubMedCentral

Carnero Contentti E, Soto de Castillo I, Daccach Marques V, et al. Application of the 2015 diagnostic criteria for neuromyelitis optica spectrum disorders in a cohort of Latin American patients. Mult Scler Relat Disord. 2018;20:109–14. https://doi.org/10.1016/j.msard.2018.01.001.CrossRefPubMed

10.

Fragoso YD, Sousa NAC, Alves-Leon SV, et al. Clinical characteristics of 153 Brazilian patients with neuromyelitis optica spectrum disorder (NMOSD). Mult Scler Relat Disord. 2018;27:392–6. https://doi.org/10.1016/j.msard.2018.11.031.CrossRefPubMed

11.

Seok JM, Cho HJ, Ahn SW, et al. Clinical characteristics of late-onset neuromyelitis optica spectrum disorder: a multicenter retrospective study in Korea. Mult Scler. 2017;23(13):1748–56. https://doi.org/10.1177/1352458516685416.CrossRefPubMed

12.

Krumbholz M, Hofstadt-van Oy U, Angstwurm K, et al. Very late-onset neuromyelitis optica spectrum disorder beyond the age of 75. J Neurol. 2015;262(5):1379–84. https://doi.org/10.1007/s00415-015-7766-8.CrossRefPubMedPubMedCentral

13.

Suchdev K, Razmjou S, Venkatachalam P, Khan OA, Mohamed W, Ibrahim MS. Late onset neuromyelitis optica mimicking an acute stroke in an elderly patient. J Neuroimmunol. 2017;309:1–3. https://doi.org/10.1016/j.jneuroim.2017.04.006.CrossRefPubMed

14.

Souza CS, Brooks JB, Oliveira CL, Fragoso YD. Neuromyelitis optica with very late onset. Arq Neuropsiquiatr. 2013;71(8):556–7. https://doi.org/10.1590/0004-282X20130077.CrossRefPubMed

15.

Otani T, Irioka T, Takahashi YK, et al. Two cases of late-onset neuromyelitis optica spectrum disorder initially presenting with isolated cerebral white matter lesions. e-Neurol Sci. 2018;13:35–7. https://doi.org/10.1016/j.ensci.2018.11.008.CrossRef

16.

Campbell A, Ogundipe OA. Neuromyelitis optica spectrum disorder presenting in an octogenarian. BMJ Case Rep. 2018. https://doi.org/10.1136/bcr-2018-225601.CrossRefPubMedPubMedCentral

17.

Amorim TA, Rocha-Filho PA. Neuromyelitis optica of very late onset: one more case. Arq Neuropsiquiatr. 2014;72(2):173. https://doi.org/10.1590/0004-282X20130211.CrossRefPubMed

18.

Lefaucheur R, Bourre B, Ahtoy P, et al. Neuromyelitis optica with very late onset. J Am Geriatr Soc. 2011;59(6):1138–40. https://doi.org/10.1111/j.1532-5415.2011.03435.x.CrossRefPubMed

19.

Collongues N, Marignier R, Jacob A, et al. Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset. Mult Scler. 2014;20(8):1086–94. https://doi.org/10.1177/1352458513515085.CrossRefPubMed

20.

Mao Z, Yin J, Zhong X, et al. Late-onset neuromyelitis optica spectrum disorder in AQP4-seropositive patients in a Chinese population. BMC Neurol. 2015;15:160. https://doi.org/10.1186/s12883-015-0417-y.CrossRefPubMedPubMedCentral

21.

Kurtzke JF. Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology (Cleveland). 1983;33:1444–52.CrossRef

22.

Lennon VA, Wingerchuk DM, Kryzer TJ, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. 2004;364(9451):2106–12.CrossRefPubMed

23.

Hollen C, Suhaib O, Farrow A, Sidorov E. Elderly-onset neuromyelitis optica spectrum disorder with pre-existing prednisone allergy. Case Rep Neurol. 2018;10(1):25–8. https://doi.org/10.1159/000485120.CrossRefPubMedPubMedCentral

24.

Montero N, Pérez-Sáez MJ, Pascual J, et al. Immunosuppression in the elderly renal allograft recipient: a systematic review. Transpl Rev (Orlando). 2016;30(3):144–53. https://doi.org/10.1016/j.trre.2016.05.001.CrossRef

25.

Sailler L. Rituximab off label use for difficult-to-treat auto-immune diseases: reappraisal of benefits and risks. Clin Rev Allergy Immunol. 2008;34(1):103–10. https://doi.org/10.1007/s12016-007-8020-7.CrossRefPubMed

Title: Late Onset of Neuromyelitis Optica Spectrum Disorders
Authors: Yara Dadalti Fragoso
Heloisa Helena Ruocco
Ronaldo Maciel Dias
Hideraldo Cabeça
Ricardo Gonçalves
Nise A. de Carvalho Sousa
Caroline Vieira Spessotto
Carlos Bernardo Tauil
Soniza Vieira Alves-Leon
Sidney Gomes
Marcus Vinicius M. Gonçalves
Suzana C. Nunes Machado
Andrea Anacleto
Eber Castro Correa
Maria Lucia V. Pimentel
Gutemberg Augusto C. Santos
Publication date: 01-12-2019
Publisher: Springer Healthcare
Keywords: Azathioprine
Optic Neuritis
Neuromyelitis Optica Spectrum Disease
Azathioprine
Published in: Neurology and Therapy / Issue 2/2019
Print ISSN: 2193-8253
Electronic ISSN: 2193-6536
DOI: https://doi.org/10.1007/s40120-019-0143-2

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Late Onset of Neuromyelitis Optica Spectrum Disorders

Abstract

Introduction

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Introduction

Methods

Results

Conclusion

Please log in to get access to this content

Other articles of this Issue 2/2019

Long-Term Observation of the Safety and Effectiveness of Enzyme Replacement Therapy in Japanese Patients with Pompe Disease: Results From the Post-marketing Surveillance

Correction to: Abstracts from the 5th International Porto Congress of Multiple Sclerosis

Long-Term Effect of Immediate Versus Delayed Fingolimod Treatment in Young Adult Patients with Relapsing–Remitting Multiple Sclerosis: Pooled Analysis from the FREEDOMS/FREEDOMS II Trials

Acknowledgement to Authors, Referees and Readers 2019

Modeling the Choice Between Risperidone Long-Acting Injectable and Generic Risperidone from the Perspective of a Japanese Hospital

Two Sides to Every Story: Perspectives from Four Patients and a Healthcare Professional on Multiple Sclerosis Disease Progression